Objective: Synthesize available evidence on cognitive, behavioral, and clinical severity measures in PSP and CBS.

Background: Cognitive and behavioral impairment are key sources of disability in tauopathies such as Progressive Supranuclear Palsy (PSP) and Corticobasal Syndrome (CBS), yet evidence on their assessment and progression is limited. Despite multiple tools to evaluate “subcortical” dementia, early subtle deficits are often considered nonspecific.

Method: Systematic literature searches identified studies reporting summary-level data (sample size, mean, standard deviation) for cognitive, behavioral, and clinical severity measures in PSP and CBS. Random-effects meta-analyses (REML) were conducted for outcomes reported in at least four independent studies. Heterogeneity was assessed using Q and I². Small-study effects, as an indicator of potential publication bias and effect size inflation in smaller samples, were explored using funnel plots and Egger’s regression. 

Results: Cognitive screening measures were the most consistently reported outcomes,particularly Mini Mental State Examintion (MMSE, n=72), Montrel Cognitive Assessment (MOCA, n=31), and Addenbrooke Cognitive Examination (ACE, n=26) in PSP, whereas substantially fewer measures were meta-analyzable in CBS. REML yielded stable pooled mean estimates for MMSE, MOCA, and ACE in PSP. Between-study heterogeneity was consistently high. Sensitivity analyses based on leave-one-out procedures identified few highly influential studies, with pooled estimates remaining largely stable after exclusion of these studies. Egger’s regression indicated evidence of small-study effects for selected outcomes, suggesting potential publication bias or exaggerated effects in smaller studies, while other measures showed no significant asymmetry. Meta-regressions suggested that greater clinical severity, lower education level and higher age were associated individually with worse cognitive performance.

Conclusion: This meta-analysis provides a quantitative overview of the available evidence of cognitive and behavioral outcomes in PSP and CBS. Despite substantial heterogeneity, pooled estimates were feasible and robust for key global cognitive screening measures, particularly MMSE, MOCA, and ACE. These findings highlight important gaps in outcome reporting and underscore the need for harmonized assessment approaches in future studies.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/a-systematic-review-and-meta-analysis-on-cognitive-and-behavioral-impairments-in-primary-tauopathies/